Despite years of intensive research, the understanding of Parkinson's disease (PD) is still rudimentary. Genetic causes of rare familial cases have offered venues of investigation, and interestingly, have strengthened the case for a mitochondrial dysfunction in the pathogenesis of PD. Mouse models, where these and other mitochondrial-related genes are affected are helping not only in understanding PD, but also in providing a powerful tool to test therapeutics. In this review, we will discuss the different characteristics of these mouse models. This article is part of a Special Issue entitled ‘Mitochondrial function and dysfunction in neurodegeneration’.